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Case Reports in Oncological Medicine
Volume 2016 (2016), Article ID 3161768, 6 pages
http://dx.doi.org/10.1155/2016/3161768
Case Report

Systemic Mastocytosis with Smoldering Multiple Myeloma: Report of a Case

1Department of Medicine, Staten Island University Hospital, Northwell Health, 475 Seaview Avenue, Staten Island, NY 10305, USA
2Department of Hematology/Oncology, Staten Island University Hospital, Northwell Health, 475 Seaview Avenue, Staten Island, NY 10305, USA
3Department of Pathology, Staten Island University Hospital, Northwell Health, 475 Seaview Avenue, Staten Island, NY 10305, USA

Received 10 March 2016; Accepted 4 May 2016

Academic Editor: Junya Kuroda

Copyright © 2016 Sassine Ghanem et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Systemic mastocytosis (SM) is a disease characterized by a clonal infiltration of mast cells affecting various tissues of the body. It is grouped into six different subtypes according to the World Health Organization classification. It is called indolent systemic mastocytosis (ISM) when there is no evidence of end organ dysfunction, while the presence of end organ dysfunction defines aggressive systemic mastocytosis (ASM). When SM coexists with a clonal hematological disorder, it is classified as systemic mastocytosis with associated clonal hematological nonmast cell lineage disease (SM-AHNMD). Over 80% of SM-AHNMD cases involve disorders of the myeloid cell lines. To our knowledge, there are only 8 reported cases to date of SM associated with a plasma cell disorder. We report a patient with ISM who was found to have concomitant smoldering multiple myeloma. His disease later progressed to ASM. We discuss this rare association between SM and a plasma cell disorder, and potential common pathophysiologic mechanisms linking the two disorders will be reviewed. We also discuss prognostic factors in SM as well as the management options considered during the evolution of the patient’s disease.